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{{CMG}} '''Associate Editor-In-Chief:''' [[User:KeriShafer|Keri Shafer, M.D.]] [mailto:kshafer@bidmc.harvard.edu] [[Priyamvada Singh|Priyamvada Singh, MBBS]] [[mailto:psingh13579@gmail.com]] '''Assistant Editor-In-Chief:''' [[Kristin Feeney|Kristin Feeney, B.S.]] [[mailto:kfeeney@elon.edu]]
{{CMG}} '''Associate Editor-In-Chief:'''{{Sara.Zand}}  [[User:KeriShafer|Keri Shafer, M.D.]] [mailto:kshafer@bidmc.harvard.edu] [[Priyamvada Singh|Priyamvada Singh, MBBS]] [mailto:psingh13579@gmail.com] '''Assistant Editor-In-Chief:''' [[Kristin Feeney|Kristin Feeney, B.S.]] [mailto:kfeeney@elon.edu]


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==Overview==


==Natural History==


==Complications==
==Overview==
* Irregular, fast heart rhythms ([[arrhythmias]])
Early clinical features in infants include [[ cyanosis]] of [[lips]] and [[tongue]], [[difficulty in breathing]], [[tiring easily during feeding]]. The severity of [[cyanosis]] in [[ infants]] with [[ pulmonary stenosis]] is dependent on the amount of [[ pulmonary blood flow ]]passing through [[ patent ductus arteriosus]].  After physiologic closure of [[ patent ductus arteriosus]] (PDA), the [[ cyanosis]] will be aggravated. In patients with normal [[ pulmonary blood flow]], complications of [[ heart failure]] may occur. Prognosis is generally poor without [[surgery ]] and 90% of patients will die before 10 years old.
* Chronic diarrhea (from a disease called [[protein loosing enteropathy]])
== Natural History, Complications and Prognosis ==
* [[Heart failure]]
* Fluid in the abdomen ([[ascites]]) and in the lungs ([[pleural effusion]])
* Blockage of the artificial shunt
* [[Stroke]]s and other neurological complications
* [[Sudden death]]


==Prognosis==
* Early clinical features in infants include [[ cyanosis]] of [[lips]] and [[tongue]], [[difficulty in breathing]], [[tiring easily during feeding]].
Improvement can be expected with most surgical procedures. Unexpected death may occur with heart arrhythmias.
* The severity of [[cyanosis]] in [[ infants]] with [[ pulmonary stenosis]] is dependent on the amount of [[ pulmonary blood flow ]]passing through [[ patent ductus arteriosus]].
* After physiologic closure of [[ patent ductus arteriosus]] (PDA), the [[ cyanosis]] will be aggravated.
* In patients with normal [[ pulmonary blood flow]], complications of [[ heart failure]] may occur.
* Prognosis is generally poor without [[surgery ]] and 90% of patients will die before 10 years old.
* The 15-year survival of patients with [[Fontan procedure]] is approximately %92.<ref name="MeryDe León2019">{{cite journal|last1=Mery|first1=Carlos M.|last2=De León|first2=Luis E.|last3=Trujillo-Diaz|first3=Daniel|last4=Ocampo|first4=Elena C.|last5=Dickerson|first5=Heather A.|last6=Zhu|first6=Huirong|last7=Adachi|first7=Iki|last8=Heinle|first8=Jeffrey S.|last9=Fraser|first9=Charles D.|last10=Ermis|first10=Peter R.|title=Contemporary Outcomes of the Fontan Operation: A Large Single-Institution Cohort|journal=The Annals of Thoracic Surgery|volume=108|issue=5|year=2019|pages=1439–1446|issn=00034975|doi=10.1016/j.athoracsur.2019.05.039}}</ref>
* Complications of [[Fontan procedure]] include:<ref name="FredenburgJohnson2011">{{cite journal|last1=Fredenburg|first1=Tyler B.|last2=Johnson|first2=Tiffanie R.|last3=Cohen|first3=Mervyn D.|title=The Fontan Procedure: Anatomy, Complications, and Manifestations of Failure|journal=RadioGraphics|volume=31|issue=2|year=2011|pages=453–463|issn=0271-5333|doi=10.1148/rg.312105027}}</ref>
**[[Exercise intolerance]] due to [[ventricular failure]]
**[[Pericardial effusion]] and[[ pleural effusion]], [[chylothorax]] and [[protein-losing enteropathy]] due to [[lymphatic dysfunction]]
**[[Pulmonary embolism]], blood clot formation in shunt
**[[Liver failure]] and portal hypertension as a result of increased pressure in the shunt
**[[leakage]] of the[[ anastomosis]]
**[[Pulmonary hypertension]]
**[[Right atrium]] dilation
**[[Arrhythmia]]


==References==
==References==
{{reflist|2}}
{{reflist|2}}
[[Category:Needs content]]
[[Category:Cardiovascular diseases]]
[[Category:Cardiovascular system]]
[[Category:Cardiovascular system]]
[[Category:Cardiology]]
[[Category:Cardiology]]
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[[Category:Pediatrics]]
[[Category:Pediatrics]]
[[Category:Disease]]
[[Category:Disease]]
[[Category:Needs content]]
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Latest revision as of 16:15, 8 November 2020

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor-In-Chief:Sara Zand, M.D.[2] Keri Shafer, M.D. [3] Priyamvada Singh, MBBS [4] Assistant Editor-In-Chief: Kristin Feeney, B.S. [5]



Overview

Early clinical features in infants include cyanosis of lips and tongue, difficulty in breathing, tiring easily during feeding. The severity of cyanosis in infants with pulmonary stenosis is dependent on the amount of pulmonary blood flow passing through patent ductus arteriosus. After physiologic closure of patent ductus arteriosus (PDA), the cyanosis will be aggravated. In patients with normal pulmonary blood flow, complications of heart failure may occur. Prognosis is generally poor without surgery and 90% of patients will die before 10 years old.

Natural History, Complications and Prognosis

References

  1. Mery, Carlos M.; De León, Luis E.; Trujillo-Diaz, Daniel; Ocampo, Elena C.; Dickerson, Heather A.; Zhu, Huirong; Adachi, Iki; Heinle, Jeffrey S.; Fraser, Charles D.; Ermis, Peter R. (2019). "Contemporary Outcomes of the Fontan Operation: A Large Single-Institution Cohort". The Annals of Thoracic Surgery. 108 (5): 1439–1446. doi:10.1016/j.athoracsur.2019.05.039. ISSN 0003-4975.
  2. Fredenburg, Tyler B.; Johnson, Tiffanie R.; Cohen, Mervyn D. (2011). "The Fontan Procedure: Anatomy, Complications, and Manifestations of Failure". RadioGraphics. 31 (2): 453–463. doi:10.1148/rg.312105027. ISSN 0271-5333.

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